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Clinical Trial Details — Status: Active, not recruiting

Administrative data

NCT number NCT01976182
Other study ID # LGL
Secondary ID
Status Active, not recruiting
Phase Phase 2
First received
Last updated
Start date November 26, 2013
Est. completion date May 26, 2027

Study information

Verified date February 2024
Source Rennes University Hospital
Contact n/a
Is FDA regulated No
Health authority
Study type Interventional

Clinical Trial Summary

LGL leukemia represents a rare subtype of chronic T or NK lymphoproliferative disorders. It is an indolent disease, the main hematological or autoimmune complications lead to a treatment in more than 60% of patients. Investigators set up at the University Hospital of Rennes, a database of more than 300 patients with LGL leukemia from major French services that support this disease, and published in 2010 the largest series of patients in the world (n = 229). However, the limited heterogeneity and retrospective data collected, as all previously released, makes it difficult the proposal of consensual treatment options. If first and second line treatments are based on the use of immunosuppression with methotrexate, cyclophosphamide, or cyclosporin A, no molecule has proven superiority over others. Methotrexate and cyclophosphamide are mainly used in the first line. Invetigators just have in the literature data on about 100 patients treated with either of these drugs. Combining the results of our series with those in the literature, invetigators estimate the respective overall response rate (RG) and complete response rate (CR) in 55% and 30% for methotrexate, and 60% and 50% for cyclophosphamide. Thus, there are four objective in this study : 1. to compare the respective efficacies of methotrexate and cyclophosphamide when administered as first-line therapies in patients suffering from T/NK LGL leukemia with severe neutropenia or neutropenia associated with infections, and/or anemia requiring transfusions, and/or auto-immune associated disease 2. to evaluate the percentage of patients refractory to methotrexate or cyclophosphamide for which a second line treatment is efficacious 3. to explore, in case of non-response to the first-line therapy, the efficacy of ciclosporine A, the comparison being performed with the treatment which was not administered in the first-line therapy 4. to evaluate the response rate according to the phenotypic subtype of LGL leukemia.


Description:

Large Granular Lymphocyte (LGL) leukemia is a clonal disorder involving tissue invasion of marrow, spleen and liver. Clinical presentation is dominated by recurrent infections associated with neutropenia, anemia, splenomegaly, and auto-immune diseases, particularly rheumatoid arthritis. Both T cell and NK cell subtypes of LGL leukemia are indolent disease and considered as a chronic illness and lead to a treatment in more than 60% of patients. LGL leukemia displays a chronic clinical course. Recommendations regarding therapy are similar for both subtypes. Indications for treatment include 1) severe neutropenia (ANC <500 mm3); 2) neutropenia (ANC <1500mm3) with symptomatic recurrent infections; 3) symptomatic or transfusion-dependent anemia and 4) associated autoimmune conditions requiring therapy, most often rheumatoid arthritis. There is no standard treatment for patients with LGL leukemia. The numerous case reports published do not provide a consensus for a particular treatment. All the six largest series published in the literature so far (collecting data on more than 40 patients) are retrospective. Immunosuppressive therapy remains the foundation of treatment including single three agents i.e. methotrexate, oral cyclophosphamide and ciclosporin A. However prospective trials involving large numbers of patients have not been performed and no molecule has proven superiority over others. Invetigators set up at the University Hospital of Rennes, a database of more than 300 patients with LGL leukemia from major French services that support this disease, and published in 2010 the largest series of patients in the world (n = 229). However, the limited heterogeneity and retrospective data collected, as all previously released, makes it difficult the proposal of consensual treatment options. Combining the results of our series with those of the literature, invetigators estimate that overall response rate and complete response rate are 55% and 30% with methotrexate, 60% and 50% with cyclophosphamide, and 55% and less than 20% with ciclosporine A, respectively. Thus, there are four objective in this study : 1. to compare the respective efficacies of methotrexate and cyclophosphamide when administered as first-line therapies in patients suffering from T/NK LGL leukemia with severe neutropenia or neutropenia associated with infections, and/or anemia requiring transfusions, and/or auto-immune associated disease 2. to evaluate the percentage of patients refractory to methotrexate or cyclophosphamide for which a second line treatment is efficacious 3. to explore, in case of non-response to the first-line therapy, the efficacy of ciclosporine A, the comparison being performed with the treatment which was not administered in the first-line therapy 4. to evaluate the response rate according to the phenotypic subtype of LGL leukemia.


Recruitment information / eligibility

Status Active, not recruiting
Enrollment 166
Est. completion date May 26, 2027
Est. primary completion date February 5, 2024
Accepts healthy volunteers No
Gender All
Age group 18 Years and older
Eligibility Inclusion Criteria: - Common criteria of LGL leukemia: the diagnosis is based on a chronic LGL peripheral blood expansion (>0.5x109/L), usually lasting more than 6 months - Specific criteria for T-LGL leukemia or NK-LGL lymphocytosis or chronic NK-LGL leukemia: - Specific criteria for T-LGL leukemia: - Expression of LGL surface markers compatible with an activated T-cell (commonly alpha-beta+/CD3+/CD8+/CD57+ and/or CD16+) phenotype or gamma-delta+ T cells; - Clonal rearrangement of TCR? gene using PCR or specific and clonal Vß expression using FCM. - Specific criteria for NK-LGL lymphocytosis or chronic NK LGL leukemia: - Expression of LGL surface markers compatible with a NK cell (commonly CD3-/CD8+/CD16+ and/or CD16+/CD56+) phenotype; - CD56+ or CD16+ NK cells greater than 0.75x109/L; - The term of chronic NK-LGL lymphocytosis is used for patients with chronic illness (NB: patients with massive tissue LGL infiltration of the spleen, liver and bone marrow and presenting aggressive clinical behavior are considered as having aggressive NK-LGL leukemia and should not be included). - Age above 18 years - ECOG performance status of 0-2 - Life expectancy of at least 1 year - Lack of previous treatment (except with G-CSF or transfusions) - At least one indication of treatment: - Isolated severe neutropenia (ANC <0.5x109/L) or neutropenia (ANC <1.5x109/L) with two or more infections requiring antibiotics; - Anemia (whatever the underlying mechanism: pure red cell aplasia or marrow infiltration) requiring transfusions greater than 2 units for two months prior to inclusion, or symptomatic anemia (hemoglobin <10g/dl) with impairment of the quality of life; - Associated complications such as systemic diseases or auto-immune diseases (i.e. recurrent uveitis, cutaneous vasculitis, autoimmune hemolytic anemia, idiopathic thrombocytopenic purpura, rheumatoid arthritis resistant to steroids and/or immunomodulator agents (colchicin, disulone, hydrochloroquine)) and justifying a treatment with methotrexate or cyclophosphamide - Written informed consent Exclusion Criteria: - Inability to understand or to follow study procedures - Prior or concurrent malignancy within the past 5 years except inactive nonmelanomatous skin cancer or carcinoma in situ of the cervix - Other serious medical illnesses, such as hepatic, renal, cardiac, pulmonary, neurologic, or metabolic disease that would preclude the patient's ability to tolerate methotrexate, cyclophosphamide, or ciclosporine A - Reactive LGL lymphocytosis (i.e. after viral infection) - ALAT/ASAT or alkalin phosphatases >3 times normal values - Creatinine clairance <50 ml/min - Serologic evidence of HIV, hepatitis C or hepatitis B infection - Non effective contraception - Positive pregnancy test - Nursing woman

Study Design


Related Conditions & MeSH terms

  • Large Granular Lymphocytes Leukemia
  • Leukemia

Intervention

Drug:
Methotrexate
methotrexate 10mg / m² orally once a week, (at split doses of 5 mg/m2 in the morning and 5 mg/m2 at night), that is to say 2 tablets of 2.5 mg at each take, during 4 months.
Cyclophosphamide
cyclophosphamide 100 mg orally once daily, that is to say 2 tablets of 50 mg at each take, during 4 months.

Locations

Country Name City State
France CHU Sud Amiens
France CHU Angers Angers
France Intern medecine Service - CH Antibes-Juan-les-Pins Antibes
France Hematology Service - CH Avignon Avignon
France Hematology Service - CH de la cote basque Bayonne
France hematology service - CH Beauvais Beauvais
France Hematology Service - CH Jean Minjoz Besançon
France Hematology Service - CH Beziers Beziers
France Hematology Unit - HOpital Avicienne Bobigny
France Hematology Service - CH Docteur Duchenne Boulogne sur Mer
France Hematology Service - CH de Brest Brest
France Hematology Service - CH François Baclesse Caen
France hematology Service - CH Louis Pasteur Chartres
France Centre Hospitalier de Cholet Cholet
France Hopital Inter-Armées Percy Clamart
France hematology Service - CHU Estaing Clermont-Ferrand
France Hematology Service - Civils hospital Colmar
France Hematology Service CHSF Corbeil Essonnes
France CHU Henri Mondor Lymphoid Hemopathy Unit Creteil
France Hematology Unit CH Michalon Grenoble
France Hematology Unit CHD Vendée La Roche sur Yon
France Hematology Unit CH LE MANS Le Mans
France CH Robert Boulin Libourne
France Hematology Unit CHRU Lille Lille
France Hematology Unit CHU Dupuytren Limoges
France CH de Bretagne Sud Lorient
France Hematology Unit - Institut Paoli-Calmettes Marseille
France Hematology Unit CHU La Conception Marseille
France Hematology Unit CH Meaux Meaux
France Hematology Unit CH Notre Dame Bon Secours Metz
France Hematogy Unit CHU ST ELOI Montpellier
France Hematology Unit CH E.MULLER Mulhouse
France Internal Medicine - CHU Hotel Dieu Nantes
France Oncology Unit CH Antoine Lacassagne Nice
France hematology Unit CHU Caremeau Nimes
France Hematology Unit - CHR Orleans Orleans
France AP-HP Hôpital Necker - Enfants Malades Paris
France Hematology Service - Hopital La Pitié Salpetrière Paris
France Hematology Unit - Hopital Hotel Dieu Paris
France Hematology Unit - Hopital Saint Antoine Paris
France Hematology Unit - Hopital Saint Louis Paris
France Hematology Unit Hopital Saint Jean Perpignan
France Hematology Service- CH Haut Leveque Pessac
France Hematology Unit CH LYON SUD Pierre Benite
France Hematology Unit CHU La Miletrie Poitiers
France Hematology Unit CH René DUBOS Pontoise
France CH Annecy - Hematology Service Pringy
France Hematology Unit- Hopital Robert Debré Reims
France Hematology Service - CHU of Rennes Rennes
France Hematology Unit - CH Becquerel Rouen
France Oncology Unit - Institut de cancérologie de la Loire Saint Priest en Jarez
France CH Saint Quentin Oncohematology Saint Quentin
France CH Yves Lefoll Saint-Brieuc
France Hematology Unit CHU Toulouse Toulouse
France Hematology Unit CHU Bretonneau Tours
France Hematology Unit Hopitaux de Brabois Vandoeuvre les Nancy
France Intern Medecine Unit CHBA Vannes
France Hôpital André Mignot Centre Hospitalier de Versailles Versailles

Sponsors (1)

Lead Sponsor Collaborator
Rennes University Hospital

Country where clinical trial is conducted

France, 

Outcome

Type Measure Description Time frame Safety issue
Primary Complete response (CR) The main endpoint will be the hematological CR rate evaluated after 4 months of treatment (binary endpoint). Complete response (CR) is defined as a normalization of clinical examination (disappearance of splenomegaly) and a complete normalization of blood counts (i.e., hemoglobin >12g/dL, platelets >150x109/L, ANC >1.5x109/L), lymphocytosis <4x109/L and circulating LGL in the normal range (<0.3x109/L). The number of LGL will be quantitated on blood smears. at Month 4
Secondary overall response rate (ORR) Hematological overall response rate (ORR) defined as the sum of complete responses and partial responses over the total number of patients. at Month 4, and at Month 8 and Month 12 in non-responders at Month 4
Secondary Complete response (CR) Complete response (CR) is defined as a normalization of clinical examination (disappearance of splenomegaly) and a complete normalization of blood counts (i.e., hemoglobin >12g/dL, platelets >150x109/L, ANC >1.5x109/L), lymphocytosis <4x109/L and circulating LGL in the normal range (<0.3x109/L). The number of LGL will be quantitated on blood smears. at Month 8 and Month 12
Secondary Hematological partial response (PR) Hematological partial response (PR) defined as an improvement in blood counts which do not meet criteria for complete remission (e.g., ANC increasing more than 50% and reaching more than 0.5 but less than 1.5x109/L with non-recurrence of infections, or hemoglobin level increasing more than 2 g/dL from baseline and transfusion requirements stopping without normalization of the hemoglobin level defined as hemoglobin >12g/dL). at Month 4, Month 8 and Month 12
Secondary Progressive disease Progressive disease defined as worsening of cytopenia or organomegaly or incidence of infections. at Month 4, Month 8 and Month 12
Secondary Time-to-relapse Time-to-relapse (censored variable). Relapse is defined as re-occurrence of either neutropenia or anemia (ANC <1x109/L and/or hemoglobin <10g/dL) or clonal LGL >0.5x109/L in complete responders, or as a return to baseline values of either ANC or hemoglobin in partial responders, at two monthly consecutive blood tests. from Month 4 to endpoint (in first-line treatment responders)
Secondary Time-to-relapse Time-to-relapse (censored variable). Relapse is defined as re-occurrence of either neutropenia or anemia (ANC <1x109/L and/or hemoglobin <10g/dL) or clonal LGL >0.5x109/L in complete responders, or as a return to baseline values of either ANC or hemoglobin in partial responders, at two monthly consecutive blood tests. from Month 8 to endpoint (in second-line treatment responders)
Secondary Molecular remission Molecular remission defined as the disappearance of the clonal pattern of TCR gamma multiplex rearrangement and Phenotypic remission is defined as :
For the T subtype: disappearance of the excess of CD3+/CD8+ cells (or disappearance of the LGL clone according to the specific subtype observed at inclusion);
For the NK subtype: disappearance of the excess of CD3-/CD56+ cells (or disappearance of the LGL clone according to the specific subtype observed at inclusion)
at Month 4 and Month 12 for hematological complete responders
Secondary Adverse events rate Adverse events rate Month 2, Month 4, Month 6, Month 8, Month 10, Month 12
Secondary Compliance Compliance Month 2, Month 4, Month 6, Month 8, Month 10, Month 12
Secondary relationship between the response to treatment and the phenotypic subtype Identification of a relationship between the response to treatment and the phenotypic subtype characterized by the panel of monoclonal antibodies defined in the ancillary study subsection. Day 1